Living with PCD

PCD is a progressive disorder, meaning it will get worse over time, for which no cure currently exists. For most patients, it is thankfully very slowly progressive. There have been limited clinical trials of therapies conducted for PCD, and there are no ‘approved’ treatments at this time. However, because PCD shares some features with cystic fibrosis (CF), a more widely-known lung disorder for which there are many tested and proven therapies, much of the current PCD treatment protocol mirrors that of CF. While CF-type disease management may benefit those affected by PCD as the primary focus in both disorders is to prevent disease progression through aggressive treatment of infections, it is important to remember that these disorders are genetically distinct and there is no actually evidence to suggest that what works in CF is the best option for PCD. More PCD-specific research is desperately needed to determine best practices for PCD.

Treatment

As we work to improve the evidence base on which to make recommendations about treating PCD, we rely on the expert consensus of clinical professionals with experience in diagnosing and treating PCD. At this link you will find the recommendations of the North American Genetic Disorders of Mucociliary Clearance (research) Consortium (GDMCC) and in Europe, the European Respiratory Society PCD Task Force. Please review the treatment recommendations from these groups for more information.

As the PCD population grows and a patient registry is developed, PCD-specific clinical trials will be conducted and treatment options will improve. With that being said, patients are encouraged to participate in research and clinical trials. To learn more about how you can get involved, please contact us.

Management

The best health outcomes occur when individuals with PCD are active participants in their own care and work with their healthcare team to maintain a healthy lifestyle.

Exercise

Exercise is an important part of overall health. Numerous studies demonstrate the physical, mental and emotional benefits of moderate exercise and it is especially critical for respiratory health. Exercise capacity and tolerance will vary by individual. What you do is less important than doing what you can within your personal limits on a consistent basis.

Mental Health

Chronic diseases are strongly associated with stress and/or anxiety as it relates to managing disease while trying to maintain the activities of daily living. The burden of care, anxiety missed school and work, fears about relationships and disease progression can all contribute to mental health challenges. Given the additional stresses individuals with PCD and their families face, it is not unusual for mental health issues to arise from time to time. In fact, it would be unusual if they never arose!

Mental health has a big impact on physical health. If you have concerns about the mental health of an individual with PCD or a family member, seek professional help. Dealing with chronic disease is hard. Mental health professionals can provide the support you need to optimize your health and quality of life.

PCD at School

One of the most important things you can do to help your child with PCD get the most out of their school experience is to educate his/her teacher and the school administration about the disorder. Here is a sample letter you can use with your child’s teachers and other school officials. Feel free to adapt for your personal use. For more information, click here.

PCD at Work

Many people with PCD pursue fulfilling careers and are able to support themselves through employment. However, for some the chronic infections and daily therapies required to maintain health in PCD can make full-time employment difficult to manage. As PCD lung disease progresses it may be necessary to consider disability. Parents of children with PCD can experience excessive missed work days for issues related to their children’s health, as well. For more information, click here.